We investigated the biochemical and clinical efficacy of dietary erucic acid (C22:1) therapy for X-linked adrenoleukodystrophy (ALD). In a double-blind crossover study of patients who were on chronic oleic acid (C18:1) therapy, addition of erucic acid to the diet led to a further reduction in plasma hexacosanoic acid (C26:0) concentration. We treated 12 newly diagnosed ALD patients with a diet enriched with erucic acid and oleic acid for 2 to 19 months. Mean plasma C26:0 concentration decreased to normal by 4 weeks, and the C26:0 composition of plasma sphingomyelin and phosphatidylcholine became normal by 4 months on therapy. Fatty acid analysis of postmortem tissues from 1 boy treated for 10 months suggested that dietary erucic acid entered the heart, liver, adrenal gland, and brain. Eight patients remained on treatment long enough (mean, 12 +/- 3 months) to evaluate their clinical response; 6 of these patients with moderate to advanced disease deteriorated neurologically or showed progression of white matter disease on brain magnetic resonance imaging whereas 2 mildly affected patients remained clinically stable after 10 and 19 months. No adverse effects of the diet occurred. We conclude that dietary erucic acid therapy is effective in lowering plasma C26:0 to normal in ALD patients, and may prevent further demyelination in some mildly affected boys.

Despite the significant morbidity and mortality associated with status epilepticus (SE), little is known about changes in cortical function that occur after SE. We evaluated cortical function after clinical SE using continuous EEG monitoring lasting at least 24 h in 180 patients admitted to the Medical College of Virginia Hospitals (MCVH). The major EEG patterns observed after SE were a normal record, burst suppression, after SE ictal discharge (ASIDs), periodic lateralizing epileptiform discharges (PLEDs), attenuation, focal and generalized slowing, and epileptiform discharges. Normalization of the EEG after SE was highly correlated with good outcome. The presence of burst suppression and ASIDs was highly statistically significantly associated with mortality. PLEDs were also highly correlated with mortality, but not to the same degree as burst suppression and ASIDs. In addition, these EEG patterns were still significantly correlated with morbidity and mortality when we controlled for etiology using multivariate logistic statistical analysis. Persistent ictal activity was observed in many patients despite control of clinical seizure activity, indicating the importance of EEG monitoring to determine treatment patterns after clinical seizure activity in SE is controlled. The results indicate that certain EEG patterns (normalization of the EEG, ASIDs, burst suppression and PLEDs) are useful predictors of outcome in SE in addition to etiology. EEG monitoring after control of clinical SE is important to guide treatment of SE and is a useful technique for evaluating prognosis.

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In this paper we review the physics and performance of silicon detectors passivated with wafer-scale molecular beam epitaxy (MBE) and atomic layer deposition (ALD). MBE growth of a two-dimensional (2D) doping superlattice on backside-illuminated (BSI) detectors provides nearly perfect protection from interface traps, even at trap densities in excess of 10 14 cm -2 . Superlattice-doped, BSI CMOS imaging detectors show no measurable degradation of quantum efficiency or dark current from long-term exposure to pulsed DUV lasers. Wafer-scale superlattice-doping has been used to passivate CMOS and CCD imaging arrays, fully-depleted CCDs and photodiodes, and large-area avalanche photodiodes. Superlattice-doped CCDs with ALD-grown antireflection coatings achieved world record quantum efficiency at deep and far ultraviolet wavelengths (100-300nm). Recently we have demonstrated solar-blind, superlattice doped avalanche photodiodes using integrated metal-dielectric coatings to achieve selective detection of ultraviolet light in the 200-250 nm spectral range with high out-of-band rejection.

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